Necrolytic Migratory Erythema

Key Points
Very rare rash associated with a malignant tumor of certain cells in the pancreas
Exact cause of cancer is unknown, but it is thought that genetic factors may play a role in its formation
Cutaneous symptoms consist of a rash affecting the face, abdomen, buttocks or lower extremities

Glucagonoma is a very rare, malignant tumor affecting the islet cells in the pancreas, which control the release of insulin and glucagon. Cutaneous symptoms consist of a rash, necrolytic migratory erythema, which can be crusty, scaly, or made up of rasied lesion filled with fluid. The rash can migrate, disappear, and reappear. It is accompanied by other systemic symptoms, including diarrhea, excessive thirst, frequent and sometimes nocturnal urination, increased appetite, inflammation of the mouth and tongue, and weight loss. Other indicators of the condition may include high blood sugar and intolerance to glucose.

Glucagonoma is a malignant tumor, with a predisposition to grow and worsen. The tumor also causes the increase of glucagon production. While the exact cause of glucagonoma is unknown, it is generally thought to be due to genetic factors, and a family history of endocrine neoplasia presents an increased risk for developing the condition. In many cases the condition may spread to the liver.

Differential Diagnosis (Other conditions with similar appearance)Acrodermatitis enteropathica
Diabetes Mellitus, Type 2
Bacteremia
Paraneoplastic Syndromes
Cirrhosis
Pellagra
Diabetes Mellitus, Type 1
Psoriasis

Diagnosis
Key Points
Initial diagnosis based on presentation of clinical symptoms
Additional testing will be performed to confirm diagnosis and rule out other conditions

Glucagonoma is initially based on the clinical presentation of various symptoms. Health care professionals will then perform tests, including CT scans, glucose level and tolerance tests, and serum glucagon tests to confirm diagnosis and rule out other conditions.

Treatment
Surgical removal is the generally accepted treatment
Does not respond to chemotherapy
Successful surgery on tumors limited to the pancreas increase survival rate by a large margin

As glucagonoma is a malignant, treatment will be done as soon as is possible after diagnosis and determining the extent of the condition to plot out the best course of treatment. Surgical removal of the tumor is the generally accepted treatment, and successful surgeries on those tumors which are limited to the pancreas increase the survival rate of affected persons by a large margin. The tumor, however, does not respond to chemotherapies, and further or more aggressive treatment may be required if the tumor has spread to the liver.